Juvenile and adult-onset scleroderma: Different clinical phenotypes

Adroviç, Amra; Karatemiz, G; Esatoğlu, Sinem Nihal; Yıldız, Mehmet; Şahin, Sezgin; Barut, Kenan; Ugurlu, S; Hatemi, Gülen; Kasapçopur, Özgür; Seyahi, Emire

doi:10.1016/j.semarthrit.2023.152197

Cited by 6 publications

(4 citation statements)

References 34 publications

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“…Although the juvenile form seems to mainly affect the skin and joints, the authors objectify the presence of interstitial lung disease in a significant percentage of children (30%), compared to that of adults (50.9%). Thus, although rarely found in the clinical profile, possible lung damage in children with jSSc must be considered when diagnosing the pathology or in its evolution ( 21 ). To reinforce what was stated previously, the medical literature exposes cases of jSSc that are complicated by cardio-pulmonary damage ( 43 , 44 ).…”

Section: Current Directions In Knowledgementioning

confidence: 99%

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses

Lupu,

Sasaran,

Jechel

et al. 2024

Front. Immunol.

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Connective tissue represents the support matrix and the connection between tissues and organs. In its composition, collagen, the major structural protein, is the main component of the skin, bones, tendons and ligaments. Especially at the pediatric age, its damage in the context of pathologies such as systemic lupus erythematosus, scleroderma or dermatomyositis can have a significant negative impact on the development and optimal functioning of the body. The consequences can extend to various structures (e.g., joints, skin, eyes, lungs, heart, kidneys). Of these, we retain and reveal later in our manuscript, mainly the respiratory involvement. Manifested in various forms that can damage the chest wall, pleura, interstitium or vascularization, lung damage in pediatric systemic inflammatory diseases is underdeveloped in the literature compared to that described in adults. Under the threat of severe evolution, sometimes rapidly progressive and leading to death, it is necessary to increase the popularization of information aimed at physiopathological triggering and maintenance mechanisms, diagnostic means, and therapeutic directions among medical specialists. In addition, we emphasize the need for interdisciplinary collaboration, especially between pediatricians, rheumatologists, infectious disease specialists, pulmonologists, and immunologists. Through our narrative review we aimed to bring up to date, in a concise and easy to assimilate, general principles regarding the pulmonary impact of collagenoses using the most recent articles published in international libraries, duplicated by previous articles, of reference for the targeted pathologies.

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Section: Current Directions In Knowledgementioning

confidence: 99%

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses

Lupu,

Sasaran,

Jechel

et al. 2024

Front. Immunol.

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“…Several studies have highlighted notable differences in progression between juvenile- and adult-onset SSc. According to Adrovic et al, adult patients exhibit a significantly higher frequency of interstitial lung disease than their juvenile counterparts [ 100 ]. Although no significant differences were observed regarding renal, gastrointestinal, and cardiac involvement, a significantly higher incidence of arterial hypertension was reported among adults.…”

Section: Vitamin D: Chemistry Metabolism Optimal Serum Concentration ...mentioning

confidence: 99%

“…MTX and GCS were more often used in jSSc than in adult-onset SSc, whereas no significant differences were noted regarding the use of other DMARDs. Conversely, adult patients more often required calcium channel blockers and angiotensin-converting enzyme inhibitors, potentially due to the higher incidence of organ involvement, including those affecting the cardiovascular system [ 100 , 101 , 104 ].…”

Section: Vitamin D: Chemistry Metabolism Optimal Serum Concentration ...mentioning

confidence: 99%

Can vitamin D be an adjuvant therapy for juvenile rheumatic diseases?

et al. 2023

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Vitamin D, known for its essential role in calcium and bone homeostasis, has multiple effects beyond the skeleton, including regulation of immunity and modulation of autoimmune processes. Several reports have shown suboptimal serum 25 hydroxyvitamin D [25(OH)D] levels in people with different inflammatory and autoimmune rheumatic conditions, and an association between 25(OH)D levels, disease activity and outcomes. Although most available data pertain to adults, insights often are extended to children. Juvenile rheumatic diseases (JRDs) are a significant health problem during growth because of their complex pathogenesis, chronic nature, multisystemic involvement, and long-term consequences. So far, there is no definitive or clear evidence to confirm the preventive or therapeutic effect of vitamin D supplementation in JRDs, because results from randomized controlled trials (RCTs) have produced inconsistent outcomes. This review aims to explore and discuss the potential role of vitamin D in treating selected JRDs. Medline/PubMed, EMBASE, and Scopus were comprehensively searched in June 2023 for any study on vitamin D supplementary role in treating the most common JRDs. We used the following keywords: “vitamin D” combined with the terms “juvenile idiopathic arthritis”, “juvenile systemic scleroderma”, “juvenile systemic lupus erythematosus”, “juvenile inflammatory myopathies”, “Behcet disease”, “periodic fever syndromes” and “juvenile rheumatic diseases”. Observational studies have found that serum 25(OH)D concentrations are lower in juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile systemic scleroderma, Behcet disease and proinflammatory cytokine concentrations are higher. This suggests that vitamin D supplementation might be beneficial, however, current data are insufficient to confirm definitively the complementary role of vitamin D in the treatment of JRDs. Considering the high prevalence of vitamin D deficiency worldwide, children and adolescents should be encouraged to supplement vitamin D according to current recommendations. More interventional studies, especially well-designed RCTs, assessing the dose–response effect and adjuvant effect in specific diseases, are needed to determine the potential significance of vitamin D in JRDs treatment.

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“…Легочная артериальная гипертензия развивается у 6-10% больных, преимущественно с лимитированной формой на поздних стадиях [45,47,48,50]. В отличие от взрослых, при ювенильном дебюте преобладает диффузная форма заболевания [51]. Согласно исследованию I. Foeldvari и соавт.…”

Section: ювенильная системная склеродермияunclassified

Problems of systemic connective tissue diseases of childhood in historical perspective

Kaleda,

Nikishina

2023

Naučno-praktičeskaâ revmatologiâ

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The relevance of study systemic connective tissue diseases with juvenile onset for modern medicine is determined by the trend towards their growth in the population, the difficulty of early diagnosis, the rapid development of disability and a poor life prognosis. The article presents the main achievements in the study of this group of diseases in children, with an emphasis on the most significant issues from a practical point of view related to diagnosis, classification, clinical features and modern approaches to treatment.

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Juvenile and adult-onset scleroderma: Different clinical phenotypes

Cited by 6 publications

References 34 publications

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses

Can vitamin D be an adjuvant therapy for juvenile rheumatic diseases?

Problems of systemic connective tissue diseases of childhood in historical perspective

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